Neuroscience Newsletter August 2010-Focus Normal Pressure Hydrocephalus

Welcome
Much of this issue of the newsletter is devoted to an entity that has been the source of debate for decades—normal pressure hydrocephalus. Deciding which patients actually have the disorder, and which ones would benefit from shunting, remains challenging at best. To try to help patients struggling with the possibility of this diagnosis, we have developed a multidisciplinary team that will assess each patient thoroughly, and provide the best possible guidance. Below you will read about the current approach to the diagnosis and treatment of NPH, as well as the roles played by neurology, neurosurgery, neuropsychology and neuro-imaging. I hope you find this informative and helpful.

John Halperin MD
Medical Director, Neurosciences

The Atlantic Neuroscience Institute Invitation to Submit Articles to the NeuroscienceNewsletter Neurosciences at Atlantic Health has grown tremendously in the past several years—hence the periodic updates on the department’s activities through the Neuroscience Newsleter. Our goal is to feature key programs each month, representing both Overlook and Morristown Memorial hospitals.

Articles and contributions come from all neuro related faculty and staff. To submit articles, new items and announcements, please contact Dr. Melvyn Heyes (melvyn.heyes@atlantichealth.org) with your suggestions.

John J. Halperin MD
The New Normal Pressure
Hydrocephalus Center

The ANI is pleased to announce the opening of our multidisciplinary center for the evaluation and treatment of normal pressure hydrocephalus (NPH). Drs. Edward Zampella(Neurosurgery),Pete Saur and Michelle Papka (Memory and Cognitive Disorders Program) and Roger Kurlan (Movement Disorders Program) will collaborate in the new center. NPH is one of those neurological diagnoses never to miss since it can be curable with ventricular shunting. NPH is characterized by the clinical triad of cognitive impairment (dementia), shuffling, unsteady gait, and urinary urgency or incontinence. Brain imaging shows ventricular enlargement out of proportion to cerebral atrophy. It has been estimated that up to 10% of patients with dementia have NPH. Hydrocephalus has been recognized as an illness as far back as the time of Hippocrates, and the benefit to be gained from draining spinal fluid was realized at about the same time. The utilization of an implanted drainage system is a far more recent occurance however.The first shunt systems were developed to treat hydrocephalus of infancy and childhood. Although the general principles of cerebrospinal fluid drainage (diversion) are the same, childhood and adult (normal pressure, NPH, occult) hydrocephalus are very different diseases in terms of both diagnosis and management.

T H E P A S S I O N T O L E A D
Atlantic Neuroscience Institute at Overlook and Morristown Memorial hospitals atlantichealth.org

The symptoms and signs of normal pressure hydrocephalus (NPH) are actually the reason the name for this illness is misleading. The symptoms that this hydrocephalus produces are not those that would typically be associated with acute increased intracranial pressure (ICP) in either the adult or child. The symptom triad of cognitive impairment/dementia, urinary incontinence, gait disturbance seen in patients with NPH is not only different from the headache, coma, decreased heart rate increased blood pressure seen in acute increased ICP - it also overlaps to varying degrees symptoms due to other illnesses frequently seen in patients in patients of the age group where NPH is most commonly seen. Alzheimer’disease, Parkinson’s disease and cervical spondylytic myelopathy can cause symptoms similar to NPH. Many patients may have one or more of these diseases concommittently further complicating the diagnostic process. From a neurosurgeons perspective, there are two main questions to be answered when evaluating a patient referred for evaluation of NPH. Are the patient’s symptoms due to NPH? Will a shunt improve the patients function? With recent improvements in both testing and the technology of the implanted shunt, the diagnosis and treatment of NPH is EASIER than in years past. Neither diagnosis nor treatment is foolproof yet however. Patients with the NPH symptom triad (dementia incontinence/gait disturbance) who have enlarged ventricles on either CT scan or MRI are admitted to the hospital for a 24-48 hour trial of CSF diversion via a lumbar drain. Improvement in any or all of the symptoms, especially gait, is considered indicative of a likely satisfactory outcome with implantation of a shunt. The newer shunts now have a valve system that is programmable with respect to the pressure at which the valve opens and allows CSF to drain. The surgeon can adjust the valve to optimize the pressure in the ventricles and obtain the best possible outcome. Dr. Kurlan points out that many patients with NPH are referred to movement disorders neurologists because their short-stepped, shuffling gait can resemble the gait of patients with Parkinson’s disease. Dr. Saur emphasizes the importance of considering NPH in patients with dementia since it is highly treatable while most dementing conditions are relentlessly progressive. One of our collaborating neuroradiologists, Dr. Neal Horner, notes that there have been advances in brain MRI with special imaging sequences and volumetric measurements to allow more specific diagnosis of NPH. Dr. Papka indicates that cognitive testing is helpful in distinguishing the type of dementia associated with NPH, having a “subcortical” pattern, from the “cortical” pattern typically seen with Alzheimer’s disease. Dr. Zampella is excited that certain clinical tests are showing increasing evidence of being able to predict which patients are expected to improve after shunting. One example is continuous lumbar drainage, in which CSF is slowly drained in the hospital over a 3 day period and documented improvement in measures of cognition and gait is used as a positive predictor of response to shunting. Dr. Zampella also points out the important recent technical advances in shunting for NPH. New programmable valves allow the easy adjustment of pressures so that problems with under- or over-drainage after shunting are markedly reduced. Our new center will utilize all of the latest methods in diagnosis, patient selection for shunting and treatment. Data consistently show that the best shunt responders are diagnosed and treated early in the course of NPH, so early referral is important.

T H E P A S S I O N T O L E A D
For a more complete discussion of the diagnosis of both diagnosis and treatment of NPH, as well as a discussion of the risks and benefits of shunt surgery, an appointment with Dr Zampella can be scheduled by calling 973-285-7800.
To refer a patient to the ANI NPH Center call 908-522-4983.
Edward Zampella, MD
Neuropsychological Assessment